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High-risk pulmonary arterial hypertension — SCE Respiratory MCQ

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HardPulmonary Vascular DiseaseHigh-risk pulmonary arterial hypertensionSCE Respiratory

A 29-year-old woman with known pulmonary arterial hypertension presents with syncope and worsening oedema. She is on an endothelin receptor antagonist. Blood pressure is 88/60 mmHg, JVP is elevated and echocardiography shows severe RV dysfunction. What is the most appropriate management?

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Correct answer: E — Urgent admission to a pulmonary hypertension centre for escalation including parenteral prostacyclin consideration

Syncope, hypotension and RV failure in PAH are high-risk features requiring urgent specialist admission and escalation, often including prostacyclin therapy consideration. Diuretic adjustment alone is inadequate. Beta-blockers may worsen RV output, and stopping PAH therapy without specialist input is unsafe.

Reference: ESC/ERS Pulmonary Hypertension Guideline; NHS PH service standards